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Heme Onc Royal College > Hepatoblastoma > Flashcards

Hepatoblastoma Flashcards

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1
Q

What conditions are associated with increased risk of hepatoblastoma?

A
  • familial adenomatous polyposis
  • Beckwith-Weidemann syndrome (associated with Wilms, RMS, adrenocorticocarcinoma)
  • very low birth weight
  • trisomy 18
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2
Q

What are some genetic causes of HB?

A
  • FAP
  • gardner syndrome
  • BWS
  • hemihyperplasia
  • T18
  • glycogen storage disease
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3
Q

What does Pretext staging describe?

A

The number of continuous sections which are tumour free

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4
Q

COG stage I

A

complete gross resection at diagnosis

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5
Q

COG stage II

A

complete gross resection with residual margins

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6
Q

COG stage III

A

incomplete gross resection, biopsy only, nodes or spill/rupture

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7
Q

COG stage IV

A

distant metastases

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8
Q

Treatment of standard risk HB

A

Single agent cisplatin Q 2 weeks x 6

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9
Q

How do you manage locally unresectable HB? (s/p neoadjuvant chemo)

A

hepatic transplant

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10
Q

Oto-protectant medications

A

Amifostine

STS

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11
Q

Cardioprotectants

A

Dexrazoxane

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12
Q

Treatment of low-risk pretext I/II s/p resection

A

C5V x 4 Q3wks

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13
Q

Treatment of high-risk (SCU, AFP<100 or pretext IV or metastatic)

A

SuperPLADO, cisplatin x 5, carbo/doxo x 5 Q2 weeks

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14
Q

AFP half-life?

A

5-7 days

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15
Q

what gene is mutated in familial adenomatous polyposis (FAP)?

A

APC

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16
Q

what is the most commonly mutated gene in hepatoblastoma

A

CTNNB1

17
Q

what is the SIOPEL staging system?

A

PRETEXT - based upon number of continuous uninvolved regions of liver.

PRETEXT 1 = 3 uninvolved regions
PRETEXT 2 = 2 uninvolved
PRETEXT 3 = 1 uninvolved
PRETEXT 4 = all regions involved

*there are specific annotations for additional risk factors (ie, venous, portal involvement, LN, tumour rupture, mets etc)

18
Q

What is the COG/Evan’s staging system?

A

Stage 1 - no mets, tumour completely resected

Stage 2 - no mets, microscopic residual tumour (ie, + margins, rupture etc)

Stage 3 - no mets, gross residual tumour

Stage 4 - mets

19
Q

SIOPEL risk grouping

A

Standard risk - PRETEXT I, II or III

High risk:

  • small cell undifferentiated
  • AFP < 100
  • tumour rupture
20
Q

COG risk grouping

A

very low risk = PRETEXT I or II with fetal histology with resection at diagnosis

low/standard risk = PRETEXT I or II, any histology, with resection at diagnosis

intermediate risk = PRETEXT II, III or IV unresectable at diagnosis (+V, +P or +Extrahepatic spread), SCU histology (AFP > 100)

high risk = any PRETEXT with metastasis, AFP < 100

21
Q

how do you stage a liver tumour?

A

need high-quality cross sectional imaging (MRI + contrast preferred) to determine PRETEXT group and annotation factors

22
Q

What is the cause of Beckwith-Wiedemann syndrome?

A

11p15 paternal uniparental disomy

23
Q

how do you screen for hepatoblastoma?

A

ultrasound and AFP q3 months from diagnosis to 4th birthday

24
Q

what intracellular pathway is aberrantly activated in hepatoblastoma and why

A

WNT pathway (usually related to CTNNB1 activating mutations/deletions)

25
Q

what two tumour markers are used in diagnosis/management of liver tumours?

A

AFP

beta-HCG

26
Q

what should you test for on histology if you suspect a small cell undifferentiated hepatoblastoma?

A

expression of INI1 - need to ensure it is not a rhabdoid tumour of the liver

27
Q

3 benign liver tumours which occur in young children < 3 yrs

A

hemangioendothelioma
mesenchymal hamartoma
teratoma

28
Q

3 benign liver tumours which occur in adolescents

A

focal nodular hyperplasia
adenoma
biliary cystadenoma

29
Q

what are the three main histologic groups of HB?

A
  • epithelial (fetal + embryonal)
  • well differentiated fetal
  • small cell undifferentiated
30
Q

what toxicity was seen on SIOPEL 4?

A
  • hematologic toxicity, febrile neutropenia, >50% had significant hearing loss
31
Q

COG AHEP0731 treatment of very low risk hepatoblastoma

A

upfront resection, no chemo

32
Q

COG AHEP0731 treatment of low risk hepatoblastoma

A

upfront resection, then C5V x 2 cycles

33
Q

COG AHEP0731 treatment of intermediate risk hepatoblastoma

A

C5V-D x 6-8 cycles, resection after 2-4 courses of chemo

34
Q

COG AHEP0731 treatment of high risk hepatoblastoma

A

VCR, irinotecan, temsirolimus x 2 cycles, then C5V-D x 6

Resection after 4-6 courses of chemo

35
Q

SIOPEL treatment of SR hepatoblastoma

A

cisplatin monotherapy followed by surgery (OS 95%)

36
Q

how can you improve the cisplatin-induced hearing loss in SR HB?

A

STS administered 6 hrs after cisplatin improves incidence of hearing loss without affecting OS

37
Q

What is the SIOPEL 3 HR protocol?

A

Cisplat Q 2wks alternating with Carbo/Dox Q 2 wks (Super PLADO)

Heme Onc Royal College (30 decks)

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